The 30-Year Outcomes of Tetralogy of Fallot According to Native Anatomy and Genetic Conditions.

BACKGROUND: The reported survival of tetralogy of Fallot (TOF) is > 97%. Patients with pulmonary atresia and/or genetic conditions have worse outcomes, but long-term estimates of survival and morbidity for these TOF subgroups are scarce. The objective of this study was to describe the 30-year outcomes of TOF according to native anatomy and the coexistence of genetic conditions. METHODS: The TRIVIA (Tetralogy of Fallot Research for Improvement of Valve Replacement Intervention: A Bridge Across the Knowledge Gap) study is a retrospective population-based cohort including all TOF subjects born from 1980 to 2015 in Québec. We evaluated all-cause mortality by means of Cox proportional hazards regression, and cumulative mean number of cardiovascular interventions and unplanned hospitalisations with the use of marginal means/rates models. We computed 30-year estimates of outcomes according to TOF types, ie, classic TOF (cTOF) and TOF with pulmonary atresia (TOF-PA), and the presence of genetic conditions. RESULTS: We included 960 subjects. The median follow-up was 17 years (interquartile range, 8-27). Nonsyndromic cTOF subjects had a 30-year survival of 95% and had undergone a mean of 2.8 interventions and 0.5 hospitalisations per subject. In comparison, TOF-PA subjects had a lower 30-year survival of 78% and underwent a mean of 8.1 interventions, with 4 times as many hospitalisations. The presence of a genetic condition was associated with lower survival (< 85% for cTOF and < 60% for TOF-PA) but similar numbers of interventions and hospitalisations. CONCLUSIONS: The anatomic types and the presence of genetic conditions strongly influence the long-term outcomes of TOF. We provided robust 30-year estimates for key markers of prognosis that may be used to improve risk stratification and provide more informed counselling to families.

Low prenatal detection rate of valvar pulmonary stenosis: What are we missing?

OBJECTIVES: Critical pulmonary stenosis (PS) and pulmonary atresia with intact ventricular septum (PAIVS) require urgent neonatal intervention. Since PS may be more insidious than PAIVS during gestation, we hypothesized that neonates with PS would have lower rates of prenatal detection than PAIVS. METHODS: We performed a retrospective chart review of all neonates who underwent diagnostic or interventional cardiac catheterization between 2000 and 2014 for critical PS or PAIVS. The rates of prenatal diagnosis were calculated for PS and PAIVS. Prenatal and postnatal echocardiographic data were reviewed. RESULTS: 178 patients met inclusion criteria: 91 with critical PS and 87 with PAIVS. The prenatal diagnosis rate for critical PS was lower than for PAIVS at 37% (34/91) vs 60% (52/87) (P = .003). At the time of diagnosis at a median gestational age of 25 weeks, the median TV z-score for patients with critical PS was larger than in PAIVS (-0.15 vs -3.0 P = .004). CONCLUSION: Critical PS had a lower prenatal detection rate than PAIVS, likely due to a relatively normal 4-chamber view at the time of routine second trimester screening in patients with PS. Color flow Doppler of the outflow tracts may improve detection, since outflow tracts may appear normal by 2D imaging.

Fetal cardiac interventions: Where do we stand?

Fetal cardiac intervention (FCI) is a novel and evolving technique that allows for in utero treatment of a subset of congenital heart disease. This review describes the rationale, selection criteria, technical features, and current outcomes for the three most commonly performed FCI: fetal aortic stenosis with evolving hypoplastic left heart syndrome (HLHS); HLHS with intact or restrictive atrial septum; and pulmonary atresia with intact ventricular septum, with concern for worsening right ventricular (RV) hypoplasia.

Manejo percutáneo de la atresia pulmonar con tabique interventricular íntegro y la estenosis pulmonar crítica / Percutaneous management of pulmonary atresia with intact ventricular septum and critical pulmonary stenosis

Introducción: Los neonatos afectos de atresia pulmonar con tabique interventricular íntegro y estenosis pulmonar crítica representan un espectro amplio, incluyendo aquellos con hipoplasia significativa del ventrículo derecho. La presencia de fístulas arteriales coronarias a ventrículo derecho puede ser una contraindicación para la descompresión del ventrículo derecho. El principal objetivo del presente trabajo es analizar los resultados a corto y largo plazo durante 20 años de estos pacientes, e identificar los factores diferenciales entre ambos grupos incluyendo aquellos pacientes afectos por fístulas arteriales coronarias. Pacientes y métodos: Estudio retrospectivo donde se identificaron todos los pacientes diagnosticados de atresia pulmonar con septo interventricular íntegro y estenosis pulmonar crítica entre los meses de enero de 1996 y enero de 2018. Se recogieron y analizaron las características morfológicas del ventrículo derecho, el manejo quirúrgico, la intervención percutánea y la evolución a corto y a largo plazo. Resultados: Fueron incluidos cincuenta y un pacientes. Un total de 9 (17,6%) fallecieron durante el seguimiento. Ninguno de ellos presentaba fístulas arteriales coronarias a ventrículo derecho. La mediana de seguimiento de los restantes 42 supervivientes fue de 8,9 años (rango: 1-16). La clase funcional según la New York Heart Association en la revisión más reciente fue de 1,2. Los supervivientes del grupo de estenosis pulmonar crítica presentaban una clase funcional de 1,1 y los del grupo de atresia pulmonar con tabique interventricular íntegro de 1,6. No hubo diferencias entre los pacientes que presentaban fístulas arteriales coronarias a ventrículo derecho y los que no. Conclusiones: La presencia de fístulas arteriales coronarias a ventrículo derecho no es una contraindicación para la vía biventricular. Los pacientes con estenosis pulmonar crítica presentan una mejor evolución que los afectos de atresia pulmonar con tabique interventricular íntegro. La estrategia de apertura agresiva y precoz de la válvula pulmonar tiene una buena supervivencia global correlacionada con una buena clase funcional

Introduction: Pulmonary atresia with intact ventricular septum and critical pulmonary stenosis in newborns encompasses a wide spectrum of disease, including cases with significant right ventricular hypoplasia and coronary artery to right ventricle fistulae, which may be considered a contraindication for decompression of the right ventricle. The aim of this study was to review the middle- and long-term outcomes of these patients over 20 years and identify differential factors between both groups, including patients with coronary artery fistulae. Patients and methods: We performed a descriptive retrospective study by identifying all patients that received a diagnosis of pulmonary atresia with intact ventricular septum and critical pulmonary stenosis between January 1996 and January 2018. We collected and analysed data regarding right ventricular morphology, surgical management, percutaneous intervention and medium- and long-term outcomes. Results: 51 patients were admitted. A total of 9 patients (17.6%) died during the follow up. None of the deceased patients had coronary artery to right ventricle fistulae. The median length of follow up in the 42 survivors was 8.9 years (1-16). The functional class based on the latest revision of the New York Heart Association classification was 1.2 for the overall sample. Survivors of critical pulmonary stenosis had a functional class of 1.1, and survivors of pulmonary atresia with intact ventricular septum a functional class of 1.6. There were no differences based on the presence or absence of coronary artery to right ventricle fistulae. Conclusions: Coronary artery to right ventricle fistulae may not be a contraindication for biventricular strategy. Patients with critical pulmonary stenosis had better outcomes compared to patients with pulmonary atresia with intact ventricular septum. The aggressive strategy of opening the pulmonary valve early on was associated with a good overall survival and correlated to a good functional class

Percutaneous obliteration of the right ventricle to avoid coronary damage by sinusoids in patients with pulmonary atresia intact ventricular septum during staged single ventricle palliation.

BACKGROUND AND AIMS: Suprasystemic pressure waves can damage the coronary arteries resulting in myocardial ischemia and excess early mortality. We aimed to reduce the coronary pressure wave through the sinusoids by abolishing RV volume with percutaneous devices. METHODS AND RESULTS: Four patients with PA-IVS and coronary sinusoids from the hypertensive rudimentary RV were evaluated at a median age 26.6 months (range: 2.7-51.7). Right ventricle coronary dependent flow to the left ventricular myocardium was excluded. All four patients had dual perfusion with competitive flow from the RV through the sinusoids to the coronary arteries. Devices used were: Amplatzer vascular plug II of 10-16 mm; 27 coils (diameter 5-15 mm) in the oldest patient. Right ventricular angiography after cavity obliteration showed no more significant coronary perfusion through the sinusoids. There were no complications or deaths. Only minor and transient changes in the levels of troponin were observed. Coronary angiography at pre-Fontan evaluation showed no progress of coronary abnormalities in two patients. CONCLUSION: In selected patients with functionally single left ventricle, obliteration of the hypertensive RV cavity by percutaneous devices is safe and abolishes the systolic pressure wave in coronary sinusoids. When performed early, this may halt coronary damage and avoid excess mortality.

Ductal stenting to improve pulmonary blood flow in pulmonary atresia with intact ventricular septum and critical pulmonary stenosis after balloon valvuloplasty.

OBJECTIVE: To assess the feasibility, safety, and efficiency of ductal stenting in pulmonary atresia with intact ventricular septum or critical pulmonary stenosis after balloon pulmonary valvuloplasty. BACKGROUND: Ductal stenting in pulmonary atresia with intact ventricular septum is a re-emerging and promising technique. There is little data available on its outcomes after establishing prograde pulmonary blood flow. METHODS: We retrospectively reviewed all neonates with pulmonary atresia with intact ventricular septum or critical pulmonary stenosis who underwent ductal stenting after balloon valvuloplasty. Ductal stenting was performed either in the same setting (group A) or a few days later after balloon valvuloplasty (group B). We compared the two groups. RESULTS: Eighteen coronary stents were transvenously delivered and successfully deployed in 18 newborns. There was no procedure-related mortality. The median hospital stay post-intervention was 6 days with a mean discharge oxygen saturation of 94%. Group A had a shorter overall hospital stay with a shorter overall time of irradiation but with a longer overall procedural time. On a follow-up of 18 months, no re-intervention for stent failure or overflow was undertaken. The median stent patency based on echocardiography was 12 months. CONCLUSION: Stenting the arterial duct in pulmonary atresia with intact ventricular septum or critical pulmonary stenosis is a feasible, safe, and efficient technique. It avoids surgery or long hospital stay with prostaglandin infusion. The minimal 6 months stent longevity provides a period of time long enough to decide whether the right ventricular diastolic function is normalised or Glenn surgery is still needed.

Transcatheter pulmonary valvuloplasty in neonates with pulmonary atresia and intact ventricular septum.

BACKGROUND: Transcatheter pulmonary valvuloplasty in neonates with pulmonary atresia and intact ventricular septum (PA-IVS) or duct-dependent pulmonary valve stenosis (DD-PVS) has become a reasonable alternative to surgical right ventricle decompression. AIM: To investigate mid-term outcomes following pulmonary valvuloplasty. METHODS: Sixty-five neonates with PA-IVS (n=29) or DD-PVS (n=36) (median age 4 days; mean weight 3.0kg) undergoing pulmonary valvuloplasty were reviewed retrospectively. Procedural data and clinical outcomes were assessed. RESULTS: Pulmonary valvuloplasty was successful in 59 patients (90.8%). Preterm birth, larger tricuspid valve annulus diameter and PA-IVS correlated with procedural failure. Eleven patients (18.6%) required a Blalock-Taussig shunt during early follow-up, despite valvuloplasty. These neonates had smaller tricuspid and pulmonary valve annulus Z-scores (-1.9 vs. -0.8 [p=0.04] and -2.5 vs. -0.9 [P=0.005], respectively) and a higher incidence of "bipartite" right ventricle (P=0.02). Mean follow-up was 5.4±3.3 years. Mortality after successful valvuloplasty was 8.5% (n=5). Among the 54 survivors, biventricular repair was achieved in 52 patients (96.3%), including nine with a previous Blalock-Taussig shunt. The cumulative rate of subsequent surgery (excluding Blalock-Taussig shunt) was 13.7% (95% confidence interval 6.8-26.7%) and 16.4% (95% confidence interval 8.5-30.4%) at 2 and 4 years, respectively. Secondary surgery was significantly more frequent in PA-IVS compared with DD-PVS, and in neonates with a Blalock-Taussig shunt (P=0.003 and 0.01, respectively). CONCLUSIONS: Selected neonates with DD-PVS or PA-IVS managed by transcatheter pulmonary valvuloplasty had a good mid-term outcome. In neonates with a borderline small right ventricle, a hybrid strategy with a supplementary source of pulmonary blood flow can be efficient to achieve biventricular repair.

Characterization of Left Ventricular Dysfunction by Myocardial Strain in Critical Pulmonary Stenosis and Pulmonary Atresia After Neonatal Pulmonary Valve Balloon Dilation.

Transient left ventricular (LV) dysfunction occurs in some infants born with critical pulmonary stenosis (PS) or membranous pulmonary atresia with intact ventricular septum (PAIVS) after pulmonary valve (PV) balloon dilation (BD). The cause for this is not well understood. We sought to characterize this LV dysfunction by investigating regional differences in this cohort using myocardial strain imaging. Patients who underwent neonatal (<2 weeks age) PV BD for critical PS or PAIVS from Jan, 2007 to March, 2014 with echocardiographic images suitable for strain analysis were identified; infants with ≥moderate post-BD LV dysfunction (ejection fraction <40%, n = 8) were matched 1:1 with controls who underwent PV BD but did not develop LV dysfunction. Longitudinal and circumferential global and segmental strain were analyzed before and after PV BD. For the 8 LV dysfunction cases, LV global longitudinal strain worsened after PV BD (-16% pre- vs -8% post-PV BD, p = 0.008) with similar impairment in global LV circumferential strain (-17% vs -8%, p = 0.008); there was no significant change in RV global or segmental longitudinal strain pre- vs post-PVBD. No significant pre/post-BD differences in global or circumferential strain were found in control pts. Segmental analysis of longitudinal and circumferential LV strain before and after balloon dilation in cases demonstrated decreased strain in all segments, but more pronounced and statistically significant in septal segments as compared with the free wall. In conclusion, transient LV dysfunction post-PV BD for critical PS/PAIVS is characterized by impaired global longitudinal and circumferential LV strain, with the most significant reductions in strain at the interventricular septum; longitudinal RV strain remains unchanged. These findings suggest that the mechanism of LV dysfunction post-PV BD is adverse ventricular-ventricular interactions specifically involving the interventricular septum.

Assessing the borderline ventricle in a term infant: combining imaging and physiology to establish the right course.

PURPOSE OF REVIEW: The purpose of this review is to describe the challenges associated with the diagnosis and treatment of children with borderline ventricles. A borderline ventricle is one in which there is concern that it will not be able to support its circulation. If a biventricular repair is attempted and fails, outcome is often poor. Thus, this early decision is important. RECENT FINDINGS: For the borderline right ventricle, options to add an additional source of pulmonary blood flow make the surgical strategy a bit more flexible than for patients with a borderline left ventricle. In general, outcome for a so-called one and one-half ventricle repair are generally good, though the long-term outcome and the effects of this physiology on lifelong exercise performance and quality of life remain to be seen. For the small left ventricle, often multiple surgeries are required to 'force' blood into the left ventricle and potentially help it grow. Though this strategy is successful in some, in others it results in significant residual cardiac issues including pulmonary hypertension. SUMMARY: Determining whether a patient will be better off in the long term with a marginal biventricular repair versus a Fontan circulation remains one of the most difficult problems in the field of pediatric cardiology and cardiac surgery.

Long-Term Right Ventricular Assist Device Therapy in an Adult with Pulmonary Atresia/Intact Ventricular Septum.

Durable ventricular assist device (VAD) support is uncommonly employed in adult congenital heart disease and often involves supporting a systemic right ventricle (RV). Ventricular assist device support of a subpulmonic RV is even more unusual.

A perforation procedure for pulmonary atresia with intact ventricular septum : Egyptian experience and adaptations.

BACKGROUND: Pulmonary atresia with intact ventricular septum (PA-IVS) is an uncommon disorder with significant morphological heterogeneity. The use of percutaneous radiofrequency (RF)-assisted perforation of the atretic valve and subsequent balloon dilation provides a relatively easy but expensive procedure that is expected to establish ante-grade flow through the pulmonary valve in most patients. OBJECTIVES: The aim of the study was to attempt a cost reduction by using catheters and wires readily available in our catheter laboratory. METHODS: A total of 50 patients presenting with PA-IVS to Cairo University Children's Hospital (CUCH) were taken to the catheterization laboratory for radiofrequency perforation using the Baylis RFP-100 generator (Baylis Medical, Montréal, Canada) or the stiff end of a coronary wire. A hybrid approach was used in selected cases. RESULTS: The overall success rate for valve perforation was 92% (46 cases), 80% of which had successful primary perforation (40 cases). Success correlated with both tricuspid valve (TV) annulus and pulmonary valve (PV) annulus Z-scores, with P values of 0.2 and 0.5, respectively. CONCLUSION: The management of PA-IVS is complex. This is a disease that necessitates a dedicated team and working collaboration between the cardiologists and cardiac surgeons. Cost limitation is essential in developing countries and innovative ideas to reduce costs are essential, especially if comparable success can be expected.

The effect of transport on the physiologic stability of neonates with ductal-dependent single-ventricle lesions.

OBJECTIVE: To compare the status of infants with hypoplastic left heart syndrome (HLHS) or pulmonary atresia-hypoplastic right heart (PA-HRH) before and following transport using the validated Transport Risk Index of Physiologic Stability (TRIPS) score. METHODS: In this retrospective review of infants with HLHS or PA-HRH transported to a Children's Hospital by a pediatric transport team, an increase in TRIPS score (temperature, blood pressure, respiratory status, and response to stimuli) following transport was defined as deterioration. Statistical analyses included t-test (paired and independent), χ2, and McNemar's tests for comparisons between groups with and without deterioration and before and after transport. RESULTS: Our cohort [n = 64; 39 (61%) HLHS and 25 (39%) PA-HRH] was predominantly female (61%), black (56%), and diagnosed antenatally (78%). Median transport time was 20 (10-30) min and age was <12 h in 48 (75%) infants. TRIPS scores worsened after transport in 24 (37.5%) infants, due to temperature (n = 10) or respiratory (n = 7) dysregulation. Infants who deteriorated during transport had HLH more often (83 versus 48%) and lower pH [7.27 (0.12) versus 7.33 (0.07)]. HLH was significantly predictive of deterioration during transport [OR 5.60 (95% C.I. 1.18-26.62)]. CONCLUSIONS: The physiologic deterioration in a third of infants with single ventricle following short transports is intriguing and may have implications on their optimal place of birth.

Transthoracic Balloon Pulmonary Valvuloplasty for Treatment of Congenial Pulmonary Atresia Patients with Intact Ventricular Septum.

BACKGROUND To summarize our clinical experience in performing transthoracic balloon pulmonary valvuloplasty for the treatment of patients suffering from congenial pulmonary atresia with intact ventricular septum (PA/IVS). MATERIAL AND METHODS Between April 2009 and April 2016, 38 patients with PA/IVS underwent transthoracic balloon pulmonary valvuloplasty in our hospital. All of them were combined with patent ductus arteriosus, tricuspid insufficiency, and atrial septal defect or patent foramen ovale. The valvuloplasty was performed from the right ventricular outflow tract through a median sternotomy incision under TEE guidance for all cases. RESULTS Thirty-five patients were successfully discharged, and 3 patients died after the operation. The 35 surviving patients were followed up. Spo2 in the 35 patients was 88-96% after the operation. The transpulmonary valvular gradient pressure was less than or equal to 30 mmHg in 31 patients and between 36 and 52 mmHg in the other 4 patients. After the surgery, tricuspid regurgitation was significantly reduced. We found only 4 patients with moderate regurgitation, 5 patients with mild to moderate regurgitation, and mild regurgitation in the remaining 26 patients. Five patients underwent a second-stage operation, including biventricular repair in 4 patients and ligation of ductus arteriosus in 1 patient. CONCLUSIONS The application of transthoracic balloon pulmonary valvuloplasty for the treatment of PA/IVS is minimally invasive and safe, which has great significance for improving the curative effect for this condition and reducing operation mortality.

Long-term results of percutaneous balloon valvuloplasty in neonatal critical pulmonary valve stenosis: a 20-year, single-centre experience.

Introduction Percutaneous balloon valvuloplasty is the primary treatment for critical pulmonary valve stenosis in neonates. Thus far, a few studies have reported long-term results of this technique in neonatal critical pulmonary valve stenosis. METHODS: We carried out a retrospective study of all consecutive newborns with critical pulmonary valve stenosis subjected to percutaneous balloon valvuloplasty at a single centre, between 1994 and 2014, to assess its immediate and long-term safety and efficacy. RESULTS: A total of 24 neonates presented with critical pulmonary valve stenosis. The mean diameter of the pulmonary annulus was 7 mm (±1.19); 33.3% had a dysplastic pulmonary valve, and 92% were started on prostaglandin E1 treatment. Percutaneous balloon valvuloplasty was performed at a mean age of 4.0±4.3 days using, on average, a balloon-to-pulmonary annulus ratio of 1.18 mm (with a range from 0.9 to 1.43). Immediate success was achieved in 22/24 patients (92%) with a reduction in the pulmonary transvalvular peak gradient (p<0.05) and in the right ventricle/systemic pressure ratio (p<0.05). There was one death (4%) 6 days after the procedure, and 29.2% of them had transient rhythm complications. For a mean follow-up time of 8.4 years, the re-intervention rate was 42.9%. In total, 14 re-interventions were performed in nine neonates, including surgery in six. Freedom from re-intervention was 50% at 8 years and 43% at 10 and 15 years. CONCLUSION: This series, to the best of our knowledge, has had the longest follow-up of neonates with critical pulmonary valve stenosis. Percutaneous balloon valvuloplasty is a safe and effective treatment, and in our study 75% of the patients were exclusively treated using this technique.

Outcomes After Decompression of the Right Ventricle in Infants With Pulmonary Atresia With Intact Ventricular Septum Are Associated With Degree of Tricuspid Regurgitation: Results From the Congenital Catheterization Research Collaborative.

BACKGROUND: Outcomes after right ventricle (RV) decompression in infants with pulmonary atresia with intact ventricular septum vary widely. Descriptions of outcomes are limited to small single-center studies. METHODS AND RESULTS: Neonates undergoing RV decompression for pulmonary atresia with intact ventricular septum were included from 4 pediatric centers. Primary end point was reintervention post-RV decompression; secondary end points included circulation type at latest follow-up. Ninety-nine patients (71 with pulmonary atresia with intact ventricular septum and 28 with virtual atresia) underwent RV decompression at median 3 (25th-75th, 2-5) days of age. Seventy-one patients (72%) underwent at least 1 reintervention after decompression. Median duration of follow-up was 3 years (range, 1-10). Freedom from reintervention was 51% at 1 month and 23% at 3 years. In multivariable analysis, reintervention was associated with virtual atresia (hazard ratio [HR], 0.51; 95% confidence interval [CI], 0.28-091; P=0.027), smaller RV length (HR, 0.94; 95% CI, 0.89-0.99; P=0.027), and ≤mild tricuspid regurgitation (TR; HR, 3.58; 95% CI, 2.04-6.30; P<0.001). Patients undergoing surgical shunt or ductal stent were less likely to have virtual atresia (HR, 0.36; 95% CI, 0.15-0.85; P=0.02) and more likely to have higher RV end-diastolic pressure (HR, 1.07; 95% CI, 1.00-1.15; P=0.057) and ≤mild TR (HR, 3.50; 95% CI, 1.75-7.0; P<0.001). Number of reinterventions was associated with ≤mild TR (rate ratio, 1.87; 95% CI, 1.23-2.87; P=0.0037). Multivariable analysis indicated that <2-ventricle circulation status was associated with ≤mild TR (odds ratio, 18.6; 95% CI, 5.3-65.2; P<0.001) and lower RV area (odds ratio, 0.81; 95% CI, 0.72-0.91; P<0.001). CONCLUSIONS: Patients with pulmonary atresia with intact ventricular septum deemed suitable for RV decompression have a high reintervention burden although most achieve 2-ventricle circulation. TR ≤mild at baseline is strongly associated with reintervention and <2-ventricle circulation at medium-term follow-up. Degree of baseline TR may be an important marker of long-term outcomes in this population.

Transradial approach for collateral embolisation.

Significant aortopulmonary collaterals in cyanotic CHD patients require closure immediately before definitive intracardiac repair. Traditionally, the transfemoral access has been used for this purpose; however in a few cases, selective and stable hooking of collaterals may be extremely difficult. We describe a case in which we used a new approach for collateral embolisation in a difficult situation.

Evaluating the risk factors of reintervention of neonates with PA/IVS and CPS/IVS after PBPV as initial intervention method.

OBJECTIVES: To analyze the clinical features, immediate and short-term outcome of percutaneous balloon pulmonary valvuloplasty (PBPV) of neonates with pulmonary atresia with intact ventricular septum (PA/IVS) or critical pulmonary stenosis with intact ventricular septum (CPS/IVS) who underwent PBPV as initial intervention methods to evaluate the risk factors of reintervention. METHOD: Thirty-eight neonates with PA/IVS or CPS/IVS admitted to Shanghai Xinhua Hospital between June 2009 and November 2014 who underwent PBPV as initial procedures were respectively studied. We analyzed their clinical features, the immediate and short-term outcome of PBPV, and evaluated the risk factors for reintervention. RESULTS: Thirty-eight neonates with PA/IVS and CPS/IVS who underwent PBPV as initial procedures were included in our study with a mean age of 13±8 days. Among the 12 patients who underwent reintervention, 7 patients had PA/IVS (64%) and 5 had CPS/IVS (36%). Patients with PA/IVS (p=0.005), small pulmonary valve diameter (p=0.035), and bad pulmonary valve development were important risk factors. The immediate and short-term outcome of PBPV was better in patients who did not need reintervention, and the peak-to-peak pulmonary transvalvular gradient in the 1st month after PBPV had the best capability in predicting reintervention with help of receiver operating characteristic curve and the logistic regression analysis. Days of prostaglandin E1 (PGE1) infusion after PBPV >5 days was also found to be a risk factor for reintervention. CONCLUSION: The short- and mid-term outcome of pulmonary valve perforation and PBPV in neonates with PA/IVS and CPS/IVS was favorable. Risk factors include PA/IVS, small pulmonary valve, bad short-term pulmonary valve development, the peak-to-peak pulmonary transvalvular gradient in the 1st month after procedure >42mmHg, and PGE1 infusion >5 days.